The outcome of tibial allograft reconstruction after resection of a tumour is inconsistent and has a high rate of failure. There are few reports on the use of tibial allografts in children with open growth plates. We performed 21 allograft reconstructions (16 osteoarticular, five intercalary) in 19 consecutive patients between seven and 17 years of age. Two had Ewing’s sarcoma, one an adamantinoma and 16 osteosarcoma, one with multifocal disease. Five patients have died; the other 14 were free from disease at the time of follow-up. Six surviving patients (eight allograft reconstructions) continue to have good or excellent function at a mean of 59 months (14 to 132). One patient has poor function at 31 months. The other seven patients have a good or excellent function after additional procedures including exchange of the allograft and resurfacing or revision to an endoprosthesis at a mean of 101 months (43 to 198). The additional operations were performed at a mean of 47 months (20 to 84) after the first reconstruction. With the use of allograft reconstruction in growing children, joints and growth plates may be preserved, at least partially. Although our results remain inconsistent, tibial allograft reconstruction in selected patients may restore complete and durable function of the limb.
Aims. The epiphyseal approach to a chondroblastoma of the intercondylar notch of a
The ulna is an extremely rare location for primary bone tumours of the elbow in paediatrics. Although several reconstruction options are available, the optimal reconstruction method is still unknown due to the rarity of proximal ulna tumours. In this study, we report the outcomes of osteoarticular ulna allograft for the reconstruction of proximal ulna tumours. Medical profiles of 13 patients, who between March 2004 and November 2021 underwent osteoarticular ulna allograft reconstruction after the resection of the proximal ulna tumour, were retrospectively reviewed. The outcomes were measured clinically by the assessment of elbow range of motion (ROM), stability, and function, and radiologically by the assessment of allograft-host junction union, recurrence, and joint degeneration. The elbow function was assessed objectively by the Musculoskeletal Tumor Society (MSTS) score and subjectively by the Toronto Extremity Salvage Score (TESS) and Mayo Elbow Performance Score (MEPS) questionnaire.Aims
Methods
Osteosarcoma is the most common primary bone malignancy among children and adolescents. We investigated whether benzamil, an amiloride analogue and sodium-calcium exchange blocker, may exhibit therapeutic potential for osteosarcoma in vitro. MG63 and U2OS cells were treated with benzamil for 24 hours. Cell viability was evaluated with the MTS/PMS assay, colony formation assay, and flow cytometry (forward/side scatter). Chromosome condensation, the terminal deoxynucleotidyl transferase dUTP nick end labelling (TUNEL) assay, cleavage of poly-ADP ribose polymerase (PARP) and caspase-7, and FITC annexin V/PI double staining were monitored as indicators of apoptosis. Intracellular calcium was detected by flow cytometry with Fluo-4 AM. The phosphorylation and activation of focal adhesion kinase (FAK) and signal transducer and activator of transcription 3 (STAT3) were measured by western blot. The expression levels of X-linked inhibitor of apoptosis protein (XIAP), B-cell lymphoma 2 (Bcl-2), B-cell lymphoma-extra large (Bcl-xL), SOD1, and SOD2 were also assessed by western blot. Mitochondrial status was assessed with tetramethylrhodamine, ethyl ester (TMRE), and intracellular adenosine triphosphate (ATP) was measured with BioTracker ATP-Red Live Cell Dye. Total cellular integrin levels were evaluated by western blot, and the expression of cell surface integrins was assessed using fluorescent-labelled antibodies and flow cytometry.Aims
Methods
Osteoarticular reconstruction of the distal femur in childhood has the advantage of preserving the tibial physis. However, due to the small size of the distal femur, matching the host bone with an osteoarticular allograft is challenging. In this study, we compared the outcomes and complications of a resurfaced allograft-prosthesis composite (rAPC) with those of an osteoarticular allograft to reconstruct the distal femur in children. A retrospective analysis of 33 skeletally immature children with a malignant tumour of the distal femur, who underwent resection and reconstruction with a rAPC (n = 15) or osteoarticular allograft (n = 18), was conducted. The median age of the patients was ten years (interquartile range (IQR) 9 to 11) in the osteoarticular allograft group and nine years (IQR 8 to 10) in the rAPC group (p = 0.781). The median follow-up of the patients was seven years (IQR 4 to 8) in the osteoarticular allograft group and six years (IQR 3 to 7) in the rAPC group (p = 0.483). Limb function was evaluated using the Musculoskeletal Tumor Society (MSTS) score.Aims
Methods
While a centralized system for the care of patients with a sarcoma has been advocated for decades, regional variations in survival remain unclear. The aim of this study was to investigate regional variations in survival and the impact of national policies in patients with a soft-tissue sarcoma (STS) in the UK. The study included 1,775 patients with a STS who were referred to a tertiary sarcoma centre. The geographical variations in survival were evaluated according to the periods before and after the issue of guidance by the National Institute for Health and Care Excellence (NICE) in 2006 and the relevant evolution of regional management.Aims
Methods
In skeletally immature patients, resection of
bone tumours and reconstruction of the lower limb often results
in leg-length discrepancy. The Stanmore non-invasive extendible
endoprosthesis, which uses electromagnetic induction, allows post-operative
lengthening without anaesthesia. Between 2002 and 2009, 55 children
with a mean age of 11.4 years (5 to 16) underwent reconstruction
with this prosthesis; ten patients (18.2%) died of disseminated
disease and one
Urgent referral to a specialist centre for patients with a soft-tissue sarcoma (STS) has been recommended by the National Institute for Health and Care Excellence (NICE) in the UK since 2006. However, the impact of this recommendation on the prognosis for these patients remains unclear. We aimed to determine the impact of the NICE guidelines on the disease-specific survival (DSS) of patients with an STS. A total of 2,427 patients with an STS referred to a supraregional centre in the ten-year periods before (n = 1,386) and after (n = 1,041) the issue of the NICE guidelines were evaluated.Aims
Methods
Aneurysmal bone cysts (ABCs) are locally aggressive lesions typically found in the long bones of children and adolescents. A variety of management strategies have been reported to be effective in the treatment of these lesions. The purpose of this review was to assess the effectiveness of current strategies for the management of primary ABCs of the long bones. A systematic review of the published literature was performed to identify all articles relating to the management of primary ABCs. Studies required a minimum 12-month follow-up and case series reporting on under ten participants were not included.Aims
Methods
Introduction. Following bone tumour resection, lower limb reconstruction results in leg-length discrepancy in skeletally immature patients. Previously, minimally invasive endoprostheses have been associated with a high risk of complications including joint stiffness, nerve injury, aseptic loosening and infection. The purpose of this study was to examine the outcome of the Stanmore non-invasive extendible endoprostheses used in our institution between 2002 and 2009 and compare them with implants used in the past. Methods. Fifty-five children with a mean age of 11.4 years (5 to 16) underwent limb reconstruction with thirty-three distal femoral, two total femoral, eight proximal femoral and twelve proximal tibial implants. Forty-six endoprostheses were lengthened in clinic without anaesthesia using the principle of electromagnetic induction. Patients were assessed using the Musculoskeletal Tumour Society Score (MSTS) and the Toronto Extremity Salvage Score (TESS). Results. Ten children (18.2%) died of disseminated disease. One
The purpose of this study was to report the long-term results of extendable endoprostheses of the humerus in children after the resection of a bone sarcoma. A total of 35 consecutive patients treated with extendable endoprosthetic replacement of the humerus in children were included. There were 17 boys and 18 girls in the series with a median age at the time of initial surgery of nine years (interquartile range (IQR) 7 to 11).Aims
Methods
The aim of this study was to report the results of custom-made endoprostheses with extracortical plates plus or minus a short, intramedullary stem aimed at preserving the physis after resection of bone sarcomas in children. Between 2007 and 2017, 18 children aged less than 16 years old who underwent resection of bone sarcomas, leaving ≤ 5 cm of bone from the physis, and reconstruction with a custom-made endoprosthesis were reviewed. Median follow-up was 67 months (interquartile range 45 to 91). The tumours were located in the femur in 11 patients, proximal humerus in six, and proximal tibia in one.Aims
Patients and Methods
Aims
Patients and Methods
The use of a noninvasive growing endoprosthesis in the management
of primary bone tumours in children is well established. However,
the efficacy of such a prosthesis in those requiring a revision
procedure has yet to be established. The aim of this series was
to present our results using extendable prostheses for the revision
of previous endoprostheses. All patients who had a noninvasive growing endoprosthesis inserted
at the time of a revision procedure were identified from our database.
A total of 21 patients (seven female patients, 14 male) with a mean
age of 20.4 years (10 to 41) at the time of revision were included.
The indications for revision were mechanical failure, trauma or infection
with a residual leg-length discrepancy. The mean follow-up was 70
months (17 to 128). The mean shortening prior to revision was 44 mm
(10 to 100). Lengthening was performed in all but one patient with
a mean lengthening of 51 mm (5 to 140).Aims
Patients and Methods
After intercalary resection of a bone tumour from the femur,
reconstruction with a vascularized fibular graft (VFG) and massive
allograft is considered a reliable method of treatment. However,
little is known about the long-term outcome of this procedure. The
aims of this study were to determine whether the morbidity of this
procedure was comparable to that of other reconstructive techniques,
if it was possible to achieve a satisfactory functional result, and
whether biological reconstruction with a VFG and massive allograft
could achieve a durable, long-lasting reconstruction. A total of 23 patients with a mean age of 16 years (five to 40)
who had undergone resection of an intercalary bone tumour of the
femur and reconstruction with a VFG and allograft were reviewed
clinically and radiologically. The mean follow-up was 141 months
(24 to 313). The mean length of the fibular graft was 18 cm (12 to
29). Full weight-bearing without a brace was allowed after a mean
of 13 months (seven to 26).Aims
Patients and Methods
We reviewed our initial seven-year experience
with a non-invasive extendible prosthesis in 34 children with primary bone
tumours. The distal femur was replaced in 25 cases, total femur
in five, proximal femur in one and proximal tibia in three. The
mean follow-up was 44 months (15 to 86) and 27 patients (79%) remain
alive. The prostheses were lengthened by an electromagnetic induction
mechanism in an outpatient setting and a mean extension of 32 mm
(4 to 80) was achieved without anaesthesia. There were lengthening
complications in two children: failed lengthening in one and the
formation of scar tissue in the other. Deep infection developed
in six patients (18%) and local recurrence in three. A total of
11 patients required further surgery to the leg. Amputation was
necessary in five patients (20%) and a two-stage revision in another.
There were no cases of loosening, but two patients had implant breakage
and required revision. The mean Musculoskeletal Tumor Society functional
score was 85% (60% to 100%) at last known follow-up. These early
results demonstrate that the non-invasive extendible prosthesis
allows successful lengthening without surgical intervention, but
the high incidence of infection is a cause for concern.
Avascular necrosis (AVN) is a serious complication
of high-dose chemotherapy for haematological malignancy in childhood.
In order to describe its incidence and main risk factors and to
evaluate the current treatment options, we reviewed 105 children
with a mean age of 8.25 years (1 to 17.8) who had acute lymphoblastic
or acute myeloid leukaemia, or a non-Hodgkin’s lymphoma. Overall,
eight children (7.6%) developed AVN after a mean of 16.8 months (8
to 49). There were four boys and four girls with a mean age of 14.4
years (9.8 to 16.8) and a total of 18 involved sites, 12 of which
were in the femoral head. All these children were aged >
nine years
(p <
0.001). All had received steroid treatment with a mean cumulative
dose of prednisone of 5967 mg (4425 to 9599) compared with a mean
of 3943 mg (0 to 18 585) for patients without AVN (p = 0.005). No
difference existed between genders and no thrombophilic disorders
were identified. Their initial treatment included 11 core decompressions
and two bipolar hip replacements. Later, two salvage osteotomies
were done and three patients (four hips) eventually needed a total
joint replacement. We conclude that AVN mostly affects the weight-bearing
epiphyses. Its risk increases with age and higher steroid doses.
These high-risk patients may benefit from early screening for AVN. Cite this article:
We have analysed the pattern of symptoms in patients presenting with synovial sarcoma to identify factors which led to long delays in diagnosis. In 35 children, the early symptoms and the results of clinical and radiological investigation were reviewed, along with the presumed diagnoses. The duration of symptoms was separated into patient delay and doctor delay. Only half of the patients had one or more of the four clinical findings suggestive of sarcoma according to the guidance of the National Institute for Clinical Excellence at the onset of symptoms. Of the 33 children for whom data were available, 16 (48.5%) presented with a painless mass and in ten (30.3%) no mass was identified. Seven (21.2%) had an unexplained joint contracture. Many had been extensively investigated unsuccessfully. The mean duration of symptoms was 98 weeks (2 to 364), the mean patient delay was 43 weeks (0 to 156) and the mean doctor delay was 50 weeks (0 to 362). The mean number of doctors seen before referral was three (1 to 6) and for 15 patients the diagnosis was obtained after unplanned excision. Tumours around the knee and elbow were associated with a longer duration of symptoms and longer doctor delay compared with those at other sites. Delays did not improve significantly over the period of our study of 21 years, and we were unable to show that delay in diagnosis led to a worse prognosis. Our findings highlight the variety of symptoms associated with synovial sarcoma and encourage greater awareness of this tumour as a potential diagnosis in childhood.
The purpose of this study was to assess the outcome
of 15 patients (mean age 13.6 years (7 to 25)) with a primary sarcoma
of the tibial diaphysis who had undergone excision of the affected
segment that was then irradiated (90 Gy) and reimplanted with an
ipsilateral vascularised fibular graft within it. The mean follow-up was 57 months (22 to 99). The mean time to
full weight-bearing was 23 weeks (9 to 57) and to complete radiological
union 42.1 weeks (33 to 55). Of the 15 patients, seven required
a further operation, four to obtain skin cover. The mean Musculoskeletal
Society Tumor Society functional score at final follow-up was 27
out of 30 once union was complete. The functional results were comparable
with those of allograft reconstruction and had a similar rate of
complication. We believe this to be a satisfactory method of biological reconstruction
of the tibial diaphysis in selected patients.
The use of allografts for the treatment of bone tumours in children is limited by nonunion and the difficulty of finding a suitable graft. Furthermore, appositional growth can’t be expected of an allograft. We used an overlapping allograft in 11 children, with a mean age of ten years (4 to 15), with a mean follow-up of 24.1 months (20 to 33). There were five intercalary and six intra-articular resections, and the tumours were in the femur in six cases and the humerus in five. Rates of union, times to union, remodelling patterns and allograft-associated complications were evaluated. No allograft was removed due to a complication. Of the 16 junctional sites, 15 (94%) showed union at a mean of 3.1 months (2 to 5). Remodelling between host and allograft was seen at 14 junctions at a mean of five months (4 to 7). The mean Musculoskeletal Tumor Society score was 26.5 of 30 (88.3%). One case of nonunion and another with screw protrusion required re-operation. Overlapping allografts have the potential to shorten time to union, decrease rates of nonunion and have positive appositional growth effect.
